Cleft Lip and Palate

Introduction
Cleft lip and palate are the most commonly found
facial congenital anomaly, it constitute 80 percent of
orofacial cleft. Cleft of the lip and palate may occur in
isolation or involve both lip and palate together. The
incidence of cleft lip among Caucasians is higher than
the Africans, Europeans and Japanese. Incidence
among the Asians varies from 1 in 400 to 500 life-births
and 1 in 1500 to 2000 among the Americans. However,
incidence of isolated cleft palate is similar among the
races (0.50 in 1000 life-births). The left-sided cleft lip
has higher incidence than the right. But, the etiology
is unknown. The cleft lip and palate have a higher
incidence among close relatives (Figs 22.1 to 22.11).
Classification
There are different methods of classification of cleft
lip and palate. Cleft lip may be complete or incomplete
and may be associated with cleft of the alveolus and
palate (figure). The cleft of the lip anterior to incisive
foramen is called the cleft of the primary palate. This
primary cleft palate may be unilateral or bilateral and
may be complete or incomplete (figure). The cleft
passes entirely between the lateral incisor and canine
teeth and passes in a V-shaped manner. Midline cleft
lip is a rare occurrence. The cleft of the palate posterior
to incisive foramen is called secondary palate. The cleft
of the secondary palate may involve both hard and
soft palate. However, classification based on Kernahan
and Stark is simple and generally acceptable.
Kernahan and Starks Classification
1. Cleft of Primary Palate
UnilateralComplete
Incomplete
BilateralComplete
Incomplete
MidlineComplete
Incomplete
2. Cleft of Secondary Palate
Complete
Incomplete
Submucus
3. Cleft of both Primary and Secondary Palate
UnilateralComplete
Incomplete
BilateralComplete
Incomplete
MidlineComplete
Incomplete
Embryology of Cleft Lip and Palate
The face is developed from five processes as a result
of migration and proliferation of neural crest
mescenchyme. The frontonasal process, a pair of
maxillary process and a pair of mandibular arches
coalesce together around the stomodeum near the fifth
week of embryo to form the face. The formation of
olfactory pits divides the frontonasal process into
median nasal process and lateral nasal process. The
lateral nasal process forms the alae of the nose. Median
nasal process extends caudally and forms bilateral
elevations called globular process. The fusion of
globular process and maxillary process gives rise to
the formation of upper lip. The globular process fuse
in the midline to form philtrum. The triangular shaped
area in front of incisive foramen and between the four
incisor teeth constitute the premaxilla (primary
palate). The failure of fusion of the globular process
with the maxillary process or mesenchymal dehiscence
gives rise to different types of cleft lip.
Introduction Classification Embryology Etiology Anatomy Clinical
Presentation Management Neonatal Care Surgical Timing Preoperative
Preparation Anesthesia Surgery Secondary Surgery Palatal Revision
TWENTY-TWO
This chapter written by Prof D Basak, MS and Mch.
Cleft Lip and Palate
153
Fig. 22.1: Incomplete cleft lip
(unilateral)
Fig. 22.2: Complete cleft lip (unilateral) Fig. 22.3: Bilateral cleft lip and
alveolus
Fig. 22.4: Incomplete cleft palate Fig. 22.5: Incomplete cleft palate Fig. 22.6: Complete cleft palate
and alveolus
Fig. 22.7: Complete cleft palate Fig. 22.8: Bilateral complete cleft palate with
premaxilla and nasal septum
Fig. 22.9A: Millard repair of cleft lip
showing the incision line
Fig. 22.9B: Repair completed with
rotation of flap
Fig. 22.10A: VY repair of cleft
palate-showing the incision line
Fig. 22.10B: Repair of the palate
i n two l ayers wi th posteri or
reposition of flap
Synopsis of Oral and Maxillofacial Surgery
154
The palate develops from the primary palate (pre-
palate) and secondary palate (palate). The wedge-
shaped primary palate developed from globular
process gives rise to parts of the pre-maxilla, nasal tip
cartilage, nasal floor, lip, alveolus and triangular-
shaped anterior palate. The lack of mesenchymal
development of the central or lateral processes leads
to different varieties of prepalatal cleft. This is also
associated with hypoplasia of the maxillary structure.
The structure posterior to incisive foramen gives rise
to secondary palate. In a seven-week embryo, palatal
process develops from maxillary process, extends
from primary palate to tonsilar fossa, and hangs
vertically. Between the 8 to 9th weeks palatal shelve
rotates from vertical position to horizontal position
due to straightening of the neck from flexed position
and dropping down of tongue, thereby separating the
oral from nasal cavity. The fusion of primary palate
and secondary palate takes place in a Y shaped manner
and the limbs of Y passes anteriorly between the
incisor and canine teeth. The ventral 3/4th of the
secondary palate ossified to form the hard palate and
fuses with the nasal ceptum. Dorsal 1/4th of the
secondary palate does not ossify and hangs like a
curtain to form soft palate. The cleft of the palate occurs
due to failure of fusion of palatal processes or
subsequent breakdown of mesenchymal structures.
The clinical sequence of cleft palate, glossoptosis and
mandibular hypoplasia as described by Robin is a
manifestation of early embryological defect.
Etiology
The etiology of the cleft lip and palate is multifactorial
involving both genetic and environmental factors. No
single gene has been implicated to the causation of
facial cleft. The facial cleft has been associated with
varieties of genetic syndrome. Because of syndromic
association, it is imperative to search for other
congenital anomalies associated with cleft lip and
palate specially, in the head and neck. Chromosome
abnormality Trisomy D syndrome may cause cleft lip
and Wander Woud syndrome a genetic defect is
associated with lower lip defect. Experimentally, cleft
has been produced in varieties of condition due to
deficiency of vitamin A, Folic acid, Pantothenic acid,
Riboflavin and Nicotinic acid. Cleft has also been
produced by excess of vitamin A, hypoxia and
ingestion of various drugs like Nitrogen Mustard,
Nucleic Acid Antagonist, Corticosteroid and
Irradiation during pregnancy. Maternal smoking and
alcoholism have also been implicated for the
occurrence of cleft lip and palate. The unilateral cleft
lip in males has a higher hereditary background than
the incomplete cleft palate, which is more common in
females and has a low hereditary background
suggesting different causes in the development of cleft
lip and palate.
Anatomy of Cleft Lip and Palate
Cleft lip and palates are separate entities. However,
cleft lip may be associated with cleft alveolus and cleft
palate. Severity of the deformity depends on the
abnormal development of median nasal process and
maxillary process. In minor degree of cleft lip the
deformity, involve the front nasal process. Due to
absence of restraining force of orbicularis oris alveolar
segment is displaced outwards and pre-maxilla is
flaired anteriorly. The alveolar gap varies from mild
to severe with varying degrees of collapse of alveolar
arch. In bilateral cleft lip the pre-maxilla shows
marked protrusion giving a grotesque appearance.
The teeth adjacent to the cleft are angled, distorted
and lateral incisor may be absent. Nasal tip and
Fig. 22.11A: Furlows Z-plasty cleft palate
repairshowing incision line
Fig. 22.11B: Mobilization and reconstruction of flap by Z-plasty
Cleft Lip and Palate
155
columella are short with flattening of the alar cartilage
maxillary hypoplasia. In incomplete unilateral cleft
palate, nasal septum (Vomer) is attached to the uncleft
side of hard palate. In bilateral complete cleft lip and
palate, Vomer is free and septum hangs freely. The
musculature of soft palate is distorted. Levator palati
muscle is attached to the posterior age of the hard
palate instead of being directed towards the midline.
Both the tensor and levator palati are attached to the
eustachian tube causing malfunctioning of the tube.
The cleft palate is occasionally associated with
retroposition of tongue and mandibular hypoplasia,
thus causing obstruction in the air passage and
abnormality in swallowing reflex as described in
Peirre Robins sequence.
Clinical Presentation
Cleft lip and palate presents with multiple clinical
problems:
1. Facial deformity is the immediate concern to the
parents and causes psychological problem to the
mother. Parent should be properly guided and be
assured that the defect in her child is curable.
2. Sucking and eating Sucking of breast is not greatly
affected in isolated cleft lip deformity as the infant
takes the nipple and areola inside the mouth during
breast-feeding. However sucking is affected in case
of cleft palate as tongue cannot compress the nipple
against the cleft palate and negative pressure is not
created during sucking. There will be regurgitation
of feeds through the cleft palate.
3. Respiratory obstruction Isolated cleft palate
deformity may cause airway obstruction in
presence of Pierre Robins sequence due to the
falling back of the tongue and retrognathia and
may need immediate interference for oropharyn-
geal reflex to develop.
4. Speech and phonation The complete speech
mechanism is ensured by velopharyngeal closure.
Voluntary contraction of soft palate aided by tensor
and levator muscle compress the soft palate against
the nasopharynx and helps in the production of
speech. Incomplete velopharyngeal closure is the
hallmark of the cleft palate. Nasal intonation is
acquired during production of vowel sounds in-
patients with velopharyngeal incompetence and
consonant sounds are distorted.
5. Teeth Alveolar cleft interfere with the develop-
ment of incisor and canine teeth. The incisor may
be absent or even duplicated. Maxilla is hypoplastic
and smaller and alveolus on the lateral side is at a
lower level than the medial segment. Teeth on the
maxillary side becomes crowded and there may
be occlusion difficulties due to mandibular
prognathism.
6. Respiratory tract Nasal tip is depressed and
columella is short in cleft lip. Baby suffers from
recurrent upper respiratory tract infection due to
the nasal regurgitation. Otitis media is common
due to the malfunctioning of eustachian tube and
hearing may be affected.
Management
The aim of treatment of cleft lip and palate is to achieve
a. Normal appearance
b. Normal swallowing of feeds without
regurgitation
c. Free airway passage
d. Normal phonation and
e. Alignment of teeth.
The general care should be started in neonatal
period to achieve the goal.
Neonatal Care
Feeding
There is not much problems of feeding in babies with
cleft lip though parents are worried about it. Feeding
in a child with cleft palate is a definite problem as
infant is unable to suck properly due to the palatal
gap and there is regurgitation of feeds during
swallowing. However, the feeding can be maintained
if milk is delivered at posterior part of the oral cavity
by specially created artificial nipple or spoon. Special
type of feeding bottle or plastic bottle can be squeezed
to deliver the food at the back of the oral cavity. Baby
also is to be held in 450 to prevent regurgitation of
feed.
An orthodontist who can prepare a plate to cover
the gap in the palate, which helps in facilitating the
feeding, should examine the baby. The base plate can
be secured in position to help in the growth of the
hard palate. Apart from this intra, oral or extra oral
orthodontic appliances will be of great help to mould
the growth of alveolus and maxilla. Elastic head cap
traction with elastic strapping for the projecting pre-
maxilla is of utmost important procedure in new born
period. This makes the lip repair easy by decreasing
the gap between the lip and alveolus. The dynamic
Synopsis of Oral and Maxillofacial Surgery
156
palatal appliances are also sometimes required for the
expansion of the collapse maxillary arches. Care
should be taken to secure free air-passage in case of
Pierre Robins sequence where glossoptosis impeded
the swallowing reflex and causes airway obstruction.
Specialized surgical technique may be required to
keep the tongue anterior and to prevent the falling
back the tongue.
Surgical Timing
Cleft lip is traditionally repaired during 3 to 4 months
of age. However, the lip is repaired after birth in some
centers. Rule of 10s is a good guide for the lip repair-
10 weeks of age, 10 gram of hemoglobin and 10 pounds
of weight. This conditions favour safe anesthesia, good
wound healing, and the other congenital anomalies
in child can be detected by this time. In unilateral cleft
lip and palate with wide gap, lip adhesion and
simultaneous closure of soft palate is being practiced
in some centers between 6 to 8 weeks of age. Definitive
lip repair is done around 6 to 8 months. Cleft palate is
usually repaired between 6 months to 18 months
depending on the growth of the baby and surgeons
choice. Early repair seems to be results in better
speech outcome. However, repair may be delayed in
babies with respiratory problems. Majority of cleft
alveolus is repaired during cleft lip repair taking
mucoperiosteum flap from medial side. Wide alveolar
gap may require bone graft between 5 to 6 years of
age. The anterior segment of the cleft palate is usually
repaired along with the repair of the cleft lip.
Preoperative Preparation
Elective surgical procedure is undertaken when the
child is free from respiratory infection and attains a
good health. The upper respiratory infection is
controlled before the repair. A complete blood count,
Prothrombin times are routinely done before
operation. Culture from nasopharynx is carried out
in case of repeated respiratory tract infections. Blood
grouping and cross-matching is done before the repair
of the cleft palate.
Anesthesia
A safe endotracheal general anesthesia is a prerequisite
of repair of cleft lip and palate. This helps in better
closure and reduces the postoperative complications.
Monitoring is done by electrocardiogram and pulse
oxymeter. The assay of Carbon dioxide in expired air
is of added advantage. The specially designed angled
endotracheal tube (RAE Tube) is fixed in the midline
of the chin and it facilitates the introduction of mouth
gag.
Surgery
Cleft Lip Repair
The numerous techniques have been evolved to repair
the cleft lip. Early technique involves straight-line
closure. However, the modern repair involves the use
of lateral flap to fill the medial deficit. Lateral
quadrilateral flap of Le Mesurier or Tennisons
triangular flap introduce tissue in the lower medial
part to produce a pouting tubercle. However Millard
in 1955 described an advancement technique in which
lateral flap is advanced in upper medial portion with
rotation of the medial segment (Figure). It preserves
the philtrum and cupids bow. This technique is easy
and adjustment can be made during the repair and
collumelar lengthening is appropriate, the tissue loss
is minimum and scar is less promonent. In this
technique emphasis has also been given to the
mobilisation of the alar cartilage and repair of the base
of the nose. In recent technique of repair of cleft lip
emphasis has also been changed from design of flap
to mobilisation and accurate functional closure of
orbicularies oris muscle and skin is closed by Z plasty
to avoid vertical closure. The critical factors for
evaluating the success of the unilateral complete cleft
lip repair are position of the alveolar segment and
vertical height of the lateral lip segment. The pre-
surgical palatal expansion device is required in case
of wide-collapse alveolus. The alveolar cleft is repaired
by mobilisation of the mucoperiosteal flap from
medial segment. Associated anterior cleft palate is
repaired during the repair of cleft lip. The nasal
deformity is corrected mobilising the alar cartilage
depending on the severity of the deformity.
Bilateral cleft lip repair involves multiple problems
of the shortening of the columella, protrusion of
premaxilla and exflair of alar cartilage, which make
the lip repair different from unilateral cleft lip repair.
Decision regarding the staged repair or bilateral repair
in one sitting depending on the columellar length,
protrusion of premaxilla and alveolar gap. Millard
rotation flap augment the central prolabial vermillion.
Recent technique concentrates on using the entire
Cleft Lip and Palate
157
prolabium for central position. The Millard technique
or modified Manchester technique (Straight line
closure) can be used satisfactorily if the bilateral
closure is contemplated. The alveolar closure and
repair of the base of the nostril is done concurrently.
In case of short columella, lengthening of columella is
undertaken by V-Y Plasty at a later period.
Repair of Cleft Palate
Controversies exist in the surgical treatment of the cleft
palate repair regarding timing and technique of staged
versus complete repair. Early repair has a better
influence on the speech and skeletal deformities. The
basic goal of treatment is muscular closure of soft
palate and closure of gap in the hard palate. This can
be achieved by side-to-side closure across the cleft in
two layers by mobilizing the mucoperiostial flap and
relaxation incision along the alveolar margin to release
the tension in the suture line. The time old technique
of Von Langenbeck is a straightforward closure but
adequate palatal lengthening is not achieved. To
achieve the lengthening of the palate oblique anterior
incisions are made on both side of the alveolar groove
and mucoperiosteal flaps are mobilised from the hard
palate, the levator muscles are separated from free age
of hard palate and the muscles are closed in the
midline. The palatal flaps are pushed back and
repaired in V-Y arrangement. This technique of Veau-
Wardill Kilner repair helps in achieving the length of
the short palate. The addition of Z plasty in the short
palate helps in the gain of additional length. Two Z
plasties described by Furlow are made in the oral and
nasal side of the muscular and mucus tissue. The
palatal length is gained with the reconstitution of
muscle and mucosa of the soft palate. In huge gap the
palate can be repaired taking flap from the tongue and
Buccal mucosal flap.
Secondary Surgery
Alveolar Gap
The alveolar gap is generally repaired concurrently
with the cleft lip repair. But in case of huge gap bone,
grafting is required to fill up the gap. The autologus
bone graft (Rib) or costal cartilage is undertaken at
five to six years of age. This procedure helps in the
closure of residual oronasal fistula and provides
support for the subsequent eruption of teeth.
Lip and Nose
In spite of different methods of techniques of the repair
of cleft lip revisional surgery is required for scar
contracture, vermillion realignment, philtrum
lengthening. Depressed nasal tip can be corrected by
cartilage graft. columellar lengthening can be done by
V-Y plasty in cases of short columella.
Palatal Revision
The incidence of post-operative fistula is very high 10
percent to 20 percent even in experienced hands. The
common site of fistula formation is anteriorly at the
junction with the pre-maxilla and posteriorly at the
junction of the soft and hard palate. Different local
flaps are created to close the fistula. However, buccal
mucosal flap or other distant flaps may be used
depending on the site and size of the fistula. In cases
of short palate with velopharyngeal incompetence
baby suffers from nasal intonation. Musculomucosal
flaps are taken from the posterior pharyngeal wall to
lengthen the palate and to diminished the nasopharyn-
geal openings. This pharyngeal flaps can be taken
either superiorly or inferiorly based to repair with the
posterior palatal margin. This technique is improved
the nasopharyngeal incompetence and diminished the
air-leak via the nasopharyngeal openings.
FURTHER READING
1. American Cleft Palate-Craniofacial Association. Parameters
for the evaluation and treatment of patients with cleft lip/
palate or other craniofacial anomalies. Cleft Palate
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